EPILEPTIC ENCEPHALOPATHY WITH CONTINUOUS SPIKE-AND-WAVE DURING SLEEP

OVERVIEW

Epileptic encephalopathy with continuous spike-and-wave during
sleep is a syndrome characterized by continuous spike-and-wave during
sleep, seizures and progressive decline in cognitive, behavioral and
psychiatric functioning.

NOTE 'Epileptic encephalopathy' denotes the
concept that the epileptic activity itself might directly contribute
additional cognitive and behavioral impairments over those expected
from the underlying etiology alone, and that suppression of epileptic
activity might minimize this additional impairment.

Clinical context

This syndrome is characterized by onset of seizures between 2 and
12 years of age (peak 4-5 years), with epileptic encephalopathy with
continuous spike-and-wave during sleep developing 1-2 years after
seizure onset. Both sexes are affected. Antecedent and birth history are usually normal, or previous
structural brain abnormalities may be present. Head size, neurological
examination and developmental progress may be normal or reflect effects of an underlying structural brain abnormality.

A progressive decline in cognitive, behavioral and psychiatric
functioning is a cardinal symptom of this syndrome. All cognitive
domains are affected including language and communication,
temporo-spatial orientation, attention and social interaction. The
impairments are typically severe and although some improvement occurs
in time, residual deficits remain after remission of seizures and EEG
abnormality (which occurs from months to up to 7 years later).